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Ménière's disease (pronounced /meɪnˈjɛərz/[1]) is a disorder of the inner ear that can affect hearing and balance to a varying degree. It is characterized by episodes of vertigo and tinnitus and progressive hearing loss, usually in one ear. It is caused by lymphatic channel dilation,[2] affecting the drainage of endolymph. It is named after the French physician Prosper Ménière, who first reported that vertigo was caused by inner ear disorders in an article published in 1861. The condition affects people differently; it can range in intensity from being a mild annoyance to a chronic, lifelong disability.[3]

Ménière's often begins with one symptom, and gradually progresses. However, not all symptoms must be present for a doctor to make a diagnosis of the disease.[5] Several symptoms at once is more conclusive than different symptoms at separate times.[6]

Attacks of vertigo can be severe, incapacitating, and unpredictable. Attacks of vertigo last no longer than 24 hours.[7] This combines with an increase in volume of tinnitus and temporary, albeit significant, hearing loss. Hearing may improve after an attack, but often becomes progressively worse. Nausea, vomiting, and sweating sometimes accompany vertigo.

Some sufferers experience what are informally known as "drop attacks"—a sudden, severe attack of dizziness or vertigo that causes the sufferer, if not seated, to fall. Patients may also experience the feeling of being pushed or pulled (Pulsion). Some patients may find it impossible to get up for some time, until the attack passes or medication takes effect.

In addition to hearing loss, sounds can seem tinny or distorted, and patients can experience unusual sensitivity to noises (hyperacusis). Some sufferers also experience nystagmus, or uncontrollable rhythmical and jerky eye movements, usually in the horizontal plane, reflecting the essential role of non-visual balance in coordinating eye movements.

Studies done on both right and left ear sufferers show that patients with their right ear affected tend to do significantly worse in cognitive performance.[8] General intelligence was not hindered, and it was concluded that declining performance was related to how long the patient had been suffering from the disease.[9]

Attacks often come in series of a few minutes to a few hours.

Cause

The exact cause of Ménière's disease is not known, but it is believed to be related to endolymphatic hydrops or excess fluid in the inner ear. It is thought that endolymphatic fluid bursts from its normal channels in the ear and flows into other areas causing damage. This is called "hydrops". The membranous labyrinth, a system of membranes in the ear, contains a fluid called endolymph. The membranes can become dilated like a balloon when pressure increases and drainage is blocked.[10] This may be related to swelling of the endolymphatic sac or other tissues in the vestibular system of the inner ear, which is responsible for the body's sense of balance. In some cases, the endolymphatic duct may be obstructed by scar tissue, or may be narrow from birth. In some cases there may be too much fluid secreted by the stria vascularis. The symptoms may occur in the presence of a middle earinfection, head trauma or an upper respiratory tract infection, or by using aspirin, smoking cigarettes or drinking alcohol. They may be further exacerbated by excessive consumption of salt in some patients. Some have pointed out that this "central hypothesis" of Ménière's is questionable, as many people without Ménière's have evidence of increased pressure in the inner ear too.

It has also been proposed that Ménière's symptoms in many patients are caused by the deleterious effects of a herpes virus.[11][12][13]Herpesviridae are present in a majority of the population in a dormant state. It is suggested that the virus is reactivated when the immune system is depressed due to a stressor such as trauma, infection or surgery (under general anesthesia). Symptoms then develop as the virus degrades the structure of the inner ear.

Ménière's typically begins between the ages of 30 and 60 and affects men slightly more than women.[14][15]

History

Ménière's disease had been recognized prior to 1972, but it was still relatively vague and broad at the time. The American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium (AAO HNS CHE) made set criteria for diagnosing Ménière's, as well as defining two sub categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).[16]

In 1985, this list changed to alter wording, such as changing "deafness" to "hearing loss associated with tinnitus, characteristically of low frequencies" and requiring more than one attack of vertigo to diagnose.[18] Finally in 1995, the list was again altered to allow for degrees of the disease:[19]

Probable - Only one definitive episode of vertigo and the other symptoms and signs

Possible - Definitive vertigo with no associated hearing loss

Treatment

Initial treatment is aimed at both dealing with immediate symptoms and preventing recurrence of symptoms, and so will vary from patient to patient. Doctors may recommend vestibular training, methods for dealing with tinnitus, stress reduction, hearing aids to deal with hearing loss, and medication to alleviate nausea and symptoms of vertigo.[citation needed]

Several environmental and dietary changes are thought to reduce the frequency or severity of symptom outbreaks. Most patients are advised to adopt a low-sodium diet, typically one to two grams (1000–2000 mg) per day[6] at first, but diets as low as 400 mg are not uncommon.[citation needed] Patients are advised to avoid caffeine, alcohol and tobacco, all of which can aggravate symptoms of Ménière's. Some recommend avoiding aspartame.[citation needed] Patients are often prescribed a mild diuretic (sometimes vitamin B6). Many patients will have allergy testing done to see if they are candidate for allergy desensitization as allergies have been shown to aggravate Ménière's symptoms.[20]

Medication

Treatments aimed at lowering the pressure within the inner ear include antihistamines, anticholinergics, steroids, and diuretics.[6] Devices that provide transtympanic micropressure pulses (such as the Meniett) are now showing some promise and is becoming more widely used as a treatment for Ménière's.[22] The Meniett, specifically, is proven to be a safe method for reducing vertigo frequency for a majority of users.[23]

The anti-herpes virus drug Aciclovir has also been used with some success to treat Ménière's Disease.[11] The likelihood of the effectiveness of the treatment was found to decrease with increasing duration of the disease probably because viral suppression does not reverse damage. Morphological changes to the inner ear of Ménière's sufferers have also been found in which it was considered likely to have resulted from attack by a herpes simplex virus.[12] It was considered possible that long term treatment with acyclovir (greater than six months) would be required to produce an appreciable effect on symptoms. Herpes viruses have the ability to remain dormant in nerve cells by a process known as HHV Latency Associated Transcript. Continued administration of the drug should prevent reactivation of the virus and allow for the possibility of an improvement in symptoms. Another consideration is that different strains of a herpes virus can have different characteristics which may result in differences in the precise effects of the virus. Further confirmation that acyclovir can have a positive effect on Ménière's symptoms has been reported.[24]

Valium has been prescribed to help sedate the vestibular system.

Surgery

Surgery may be recommended if medical management does not control vertigo.[citation needed] Permanent surgical destruction of the balance part of the affected ear can be performed for severe cases if only one ear is affected. This can be achieved through chemical labyrinthectomy, in which a drug (such as gentamicin) that "kills" the vestibular apparatus is injected into the middle ear. Alternatively, surgeons can cut the nerve to the balance portion of the inner ear in a vestibularneurectomy, or the inner ear itself can be surgically removed (labyrinthectomy). These treatments eliminate vertigo, but because they are destructive, they are a last resort. Typically balance returns to normal after these procedures (albeit a balance system only using the unaffected side) but hearing loss may continue to progress.[6]

Surgery to decompress the endolymphatic sac has shown to be effective for temporarily relief from symptoms. Most patients see a decrease in vertigo occurrence, while their hearing may be unaffected. This treatment, however, does not address the long-term course of vertigo in Ménière's disease.[25] Danish studies even link this surgery to a very strong placebo effect, and that very little difference occurred in a 9-year followup, but could not deny the efficacy of the treatment.[26]

Progression

Meniere's disease usually starts confined to one ear but it often extends to involve both ears over time so that after 30 years, 50% of patients with Meniere's have bilateral disease (Stahle et al, 1991). There is some controversy about this statistic however; some authors suggest that the prevalence of bilaterality is as low as 17% (Silverstein, 1992).

Sufferers whose Ménière's began with one or two of the classic symptoms may develop others with time. Attacks of vertigo can become worse and more frequent over time, resulting in loss of employment, loss of the ability to drive, and inability to travel.[citation needed] Some patients become largely housebound. Hearing loss can become more profound and may become permanent. Some patients become deaf in the affected ear. Tinnitus can also worsen over time. Some patients with unilateral symptoms, as many as fifty percent by some estimates, will develop symptoms in both ears.[citation needed] Some of these will become totally deaf.

Yet the disease may end spontaneously and never repeat again.[citation needed] Some sufferers find that after eight to ten years their vertigo attacks gradually become less frequent and less severe; in some patients they disappear completely. In some patients, symptoms of tinnitus will also disappear, and hearing will stabilize (though usually with some permanent loss).[citation needed]

Migraine link

There is an increased prevalence of migraine in patients with Ménière’s disease[citation needed]. As well, migraine leads to a greater susceptibility of developing Ménière’s disease[citation needed]. The distinction between migraine-associated vertigo and Ménière’s is that migraine-associated vertigo may last for more than 24 hours.[7]

Famous sufferers and possible sufferers

In history

Alan B. Shepard, the first Americanastronaut, was diagnosed with Ménière’s disease in 1964, grounding him after only one brief spaceflight. Several years later, surgery (which was then at the experimental stage) was performed, allowing Shepard to fly to the Moon on Apollo 14.[28]

Marilyn Monroe, American actress and cultural icon was known to experience the vertigo and compromised hearing associated with Ménière’s.[29]

Charles Darwin may have suffered from Ménière’s disease.[citation needed] This idea is based on a common list of symptoms which were present in Darwin's case, such as tinnitus, vertigo, dizziness, motion sickness, vomiting, continual malaise and tiredness. The absence of hearing loss and 'fullness' of the ear (as far as known) excludes however a diagnosis of typical Ménière’s disease. Darwin himself had the opinion that most of his health problems had an origin in his 4-year bout with sea sickness. Later, he could not stand traveling by carriage, and only horse riding would not affect his health. One of the diagnoses that he received from his physicians at the time was that of "suppressed gout". The source of Darwin's illness is not known for certain. See Charles Darwin's illness for more details.

Martin Luther wrote in letters about the distresses of vertigo, and suspected Satan was the cause.[30][31]

A paper by Arenberg et al., 1990, suggested that Vincent Van Gogh, the Dutch Post-Impressionist, may have suffered from Ménière's,[33] though this is now considered conjectural.[citation needed] See Vincent van Gogh's medical condition for a discussion of the range of possible alternative diagnoses. Some believe "The Starry Night" illustrates his dizziness. It's also speculated that ear pressure and deafness could have inspired him to cut off his own ear.[citation needed]

Shawnae Jebbia, Miss USA 1998, was diagnosed with Ménière's disease while fulfilling her duties as Miss USA for the following year. Previously, she had a successful run as a fitness model/instructor on ESPN2, but this forced Jebbia into retiring from the entertainment business.[44]

Singer and actress Kristin Chenoweth has performed on stage while suffering from severe symptoms of Ménière's disease. She wrote about her experience with Ménière's in her 2009 autobiography and talked about it on "Fresh Air with Terry Gross" in April 2009.[45][46]

Referenced in skit on Late Night with Jimmy Fallon. August 8, 2009.

Both Aaron Yan and Calvin Chen from Fahrenheit are confirmed to have this disease.